Prune Belly (Eagle-Barrett) Syndrome (Short version)
What is Prune Belly syndrome?
Prune Belly syndrome, or Eagle-Barrett or triad syndrome, is a distinctive set of physical problems a child is born with.
These problems are:
- The abdominal muscles in the front are weak or missing
- Parts of the urinary tract develop abnormally
- In males (95% of cases), the testes stay up inside the abdomen and do not come down into the scrotum
Other physical problems in the infant may include spinal curvature, hip dislocations, clubfoot, respiratory or heart problems, and gastrointestinal problems.
What causes it?
The cause of the syndrome is unknown, but researchers believe that disruptions in the fetus's growth cause problems. Something blocks a part of the fetus' urinary tract, causing other parts of the tract to develop abnormally. Prune Belly syndrome occurs in 1 in 40,000 live births in the U.S.
How is it diagnosed?
Ultrasound during pregnancy can sometimes detect abnormal bladder and urinary tract development. While the fetus grows, fluid develops in its abdomen, which stretches larger and larger. The fluid is reabsorbed before birth, so when born, the infant has a sagging or wrinkled abdomen (thus the "prune belly" name).
After birth, ultrasound and X-rays can determine what type of urinary tract abnormalities are present.
Unfortunately, Prune Belly syndrome can have severe effects on the infant. 20% of babies die before birth, and 30% die from kidney problems within the first two years of life. In the remaining 50% of infants, there are varying degrees of urinary problems.
How is it treated?
The syndrome has no cure, but repairs to the urinary tract can be made. Some surgeons are attempting to correct problems in the fetus before it is born, but this has had mixed results. Children prone to urinary tract infections are given preventive antibiotics. Surgery can be done to bring a boy's testes down into his scrotum.
Sometimes, despite the best treatment, a child's kidneys will stop working (renal failure). The treatment for this is either dialysis or kidney transplantation.
Future research
Better surgical techniques, especially using laparoscopy, will make the necessary repair operations for a child with Prune Belly syndrome less complicated. As more is learned about the syndrome, treatments can be improved, and more children can be helped to survive.
Mary Kugler, MSN, RN,C Guide to Rare/Orphan Diseases